|Year : 2019 | Volume
| Issue : 2 | Page : 217-219
Cardiac metastasis of a uterine leiomyosarcoma
Joseph Ahoubim1, Bryce Sarcar1, Yazan Abdeen2
1 Arkansas College of Osteopathic Medicine, Fort Smith, AR, USA
2 Department of Pulmonary Medicine, Mercy Clinic, Fort Smith, AR, USA
|Date of Web Publication||18-Jul-2019|
Arkansas College of Osteopathic Medicine, Fort Smith, AR, 72916
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Uterine leiomyosarcoma (ULMS) is a rare malignancy of the myometrial smooth muscle that accounts for 1%–2% of cancers arising in women of postmenopausal age. ULMS is associated with significant morbidity and mortality, largely due to its ability to metastasize. The most common sites of metastasis include the lungs, bone, and liver. However, in rare cases, ULMS can metastasize to the heart and should be considered in patients with concomitant cardiovascular symptoms and known history of ULMS or risk factors of ULMS. Here, we report a case of a 74-year-old female who was diagnosed with ULMS-derived cardiac metastasis. Currently available treatment plans for cardiac metastasis involve a combination of medications such as mesna, doxorubicin, ifosfamide, and dacarbazine; radiation to the cardiac muscle; and resection of the mass. Given the significant improvement in the 5-year survival rate of patients who are promptly and accurately diagnosed, physicians should consider the possibility of cardiac metastases when patients with a history of ULMS present with cardiac abnormalities.
Keywords: Cardiac metastasis, leiomyosarcoma, uterine leiomyosarcoma
|How to cite this article:|
Ahoubim J, Sarcar B, Abdeen Y. Cardiac metastasis of a uterine leiomyosarcoma. J Nat Sc Biol Med 2019;10:217-9
| Introduction|| |
As reported by the National Cancer Institute's Surveillance, Epidemiology, and End Results Cancer Statistic Review, mesenchymal malignancies of the uterus represent 3.6% of all sarcomas diagnosed in the United States over the past four decades. Mesenchymal tumors of the uterus include endometrial stromal sarcoma, undifferentiated endometrial sarcoma, and smooth muscle tumors that include leiomyoma, smooth muscle tumors of uncertain malignant potential (STUMP), and leiomyosarcomas. Uterine leiomyosarcomas (ULMS) are a malignant subset of single-molecule toroic and are highly aggressive, with a proclivity to metastasize to remote sites within the body. Common sites of metastasis include the lungs, cranial/intracranial areas, skin/soft tissues and bone, with other sites including the thyroid, salivary gland, heart, liver, pancreas, adrenal gland, bowel, and breast. Cardiac metastasis of ULMS is extremely rare, with only 11 reported cases over the past four decades. Cardiac metastasis confers significant implications, such as heart failure and hemodynamic deterioration if left undiagnosed in ULMS patients. Here, we present the case of a 74-year-old female with a relapsing malignant ULMS that has metastasized to the heart causing both pericardial and pleural effusion.
| Case Report|| |
A 74-year-old Caucasian female presented to the emergency department with a 5-day history of progressive shortness of breath associated with progressive edema of both lower extremities and abdominal bloating. She stated there was no chest pain, nausea, diaphoresis, dizziness, fever, or chills associated with her shortness of breath. Furthermore, there was no associated cough, abdominal pain, diarrhea, or emesis and she stated her shortness of breath worsened when lying down.
Ten years before the current presentation, the patient was evaluated for a right adnexal mass and underwent a right salpingo-oophorectomy. It was later diagnosed as a large mucinous cystadenoma. She underwent a left salpingo-oophorectomy for a large myomatous uterus 6 months later. At that time, a uterine sample was sent to a tertiary center pathology department and the uterine mass was interpreted as a STUMP.
On the current presentation, the patient was in mild distress with a respiratory rate of 22, SpO2 92%, and a BP of 177/110. Her physical examination was significant for distant heart sounds, abdominal distention, and bilateral lower limb edema. Echocardiography showed large hemodynamically significant pericardial effusion with both right ventricular and right atrial collapse and inferior vena cava dilated. An abdominal and pelvic computed tomography (CT) scan showed a large pericardial effusion, with a small right pleural effusion and anterior cardiac wall density [Figure 1]. A pelvic mass involving the left adnexal area was also evident [Figure 2].
|Figure 1: Computed tomography scan of the patient showing pericardial effusion, right pleural effusion, and anterior wall density|
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|Figure 2: Computed tomography of patient showing pelvic mass in the left adnexal area|
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The patient underwent emergent left anterolateral thoracotomy, pericardial window, and biopsy of the mass on the anterior wall of the heart, which measured about 5 cm × 7 cm. Pathology sections of the anterior wall of the heart mass revealed a lesion composed of spindle and polygonal-shaped cells arranged in fascicles. Both cell shapes displayed enlarged nuclei with clumped chromatin and large amounts of eosinophilic cytoplasm [Figure 3]. There were >10 mitoses/10 high-power fields in some areas of the tumor. Immunoperoxidase stains were performed and the lesional cells were revealed to be desmin and smooth muscle actin positive [Figure 4] and [Figure 5] consistent with low-grade leiomyosarcoma. Analysis of a left pelvic mass CT-guided biopsy confirmed the same histologic characteristics for cardiac wall tumor confirming metastatic leiomyosarcoma. The oncology department was consulted, and a regimen of mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) was recommended, to be followed by radiation to cardiac muscle and radiation or surgery of bilateral adnexal masses if possible.
|Figure 3: High-power field microscopy showing spindle-shaped cells with enlarged nuclei, clumped chromatin, and large amounts of eosinophilic cytoplasm|
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|Figure 5: Smooth muscle actin-positive staining of anterior cardiac wall|
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| Discussion|| |
ULMS is a rare but aggressive tumor derived from myometrial smooth muscle. ULMS contributes to only 1%–2% of all uterine malignancies but is responsible for up to 70% of uterine sarcomas found in postmenopausal women. Survival is largely dependent on metastasis, with a 5-year survival rate of 91.2% for nonmetastatic ULMS and 41.2% for metastatic ULMS. This variability in numbers is due to the malignancy's known metastatic potential, as demonstrated by the patient in this case whose 10-year prior hysterectomy initially delayed initial metastasis of her tumor. Studies show that the most common sites for metastatic disease include the lung (74%), peritoneum (41%), bones (33%), and liver (27%). While rare, possibly due to the complex three-tiered lymphatic structure of the heart and its contractile nature, cardiac metastases have been reported in the literature  and should be considered in patients with cardiovascular symptoms and known ULMS or risk factors of the malignancy. These risk factors include postmenopausal status, prior pelvic radiation, and long-term tamoxifen use patients.
Metastatic cancer cells from ULMS may reach the heart through a lymphatic or hematogenous route; however, they most frequently find the lungs and breast as primary sites. Cardiac metastases from ULMS most commonly present as atrial or ventricle masses detected on clinical presentation.,,,,,, However, patients can present with pericardial and pleural effusion. Our patient presented to the ED with progressive shortness of breath. Physical examination showed distant heart sounds, bilateral lower extremity edema, and abdominal distention. Preoperative thoracic imaging, such as chest X-ray, echocardiogram, or CT angiogram, should be considered. Echocardiogram results for cardiac metastasis can show large hemodynamically significant pericardial effusion, pleural effusion, and pericardial masses, which is similar to what was observed in our patient.,, Standard protocol for patients with nonmetastasized ULMS includes total abdominal hysterectomy with additional salpingo-oophorectomy determined for each patient individually. In patients with confirmed extrauterine metastases to cardiac smooth muscle, treatment plans include chemotherapeutic medications with simultaneous drainage of the pericardial effusion, surgical resection, and if tolerated, radiation therapy to the cardiac muscle which showed a better prognosis in reported patients.,, The use of these chemotherapeutic medications in the form of MAID and cardiac radiation therapy was planned and offered for the patient in our case, in addition to surgical resection of the bilateral adnexal mass. When feasible, complete resection should be considered by clinicians, as this demonstrates the best prognosis. Patients with complete resection have been shown to live free of disease in the heart at follow-up.,
Given the aggressive nature of ULMS and the overall rarity of cardiac metastases, we present this case to provide further evidence for the potential of cardiac metastasis. Our case reinforces prior studies that suggest it is important for clinicians to consider ULMS metastases when patients present with myocardial lesions, particularly in a patient with a positive history of a STUMPs. Considering the improved 5-year survival rate of patients who are quickly and accurately diagnosed, it is vital for physicians to heed the potential correlation between cardiac-related symptoms and ULMS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]